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Title: The development and assessment of executive functioning in preschool children with and without sickle cell anaemia
Author: Downes, M.
ISNI:       0000 0004 7230 3895
Awarding Body: UCL (University College London)
Current Institution: University College London (University of London)
Date of Award: 2016
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Executive functioning is the ability to execute cognitive control in high-level processes such as planning or problem solving. Executive functions are susceptible to disease. Deficits have been highlighted in many school age and adolescent patient populations, including sickle cell disease. However, research with preschool children largely depends on general developmental measures, non-validated behavioural measures, and parental report, making it difficult to ascertain the extent of potential executive deficits. Sickle cell disease, the most common hereditary disorder in the United Kingdom, is a blood disorder caused by an abnormal gene for haemoglobin. These children are at a high risk of stroke, but even without stroke, they can develop deficits in executive functioning. Although executive deficits have been identified in school-age children with sickle cell disease, there has been no research in preschool children that has attempted to delineate whether these deficits are present and/or detectable at this early stage. Early detection could lead to the development of targeted interventions and improve school readiness. Executive tasks, including an ERP study, were developed, piloted, and validated with typically developing preschool children. One of the tasks was also normed in a larger population (N=166, 3-5 years). Tasks developed during this thesis that were considered valid were administered to children with sickle cell disease and a matched comparison group, alongside other recently developed and standardised assessments of executive functioning. Children with sickle cell disease obtained poorer scores on behavioural executive tasks with differences also observed for underlying neural correlates on the ERP task. However, these differences did not translate to parental report. Specific deficits, detected by behavioural measures, may not yet have translated into everyday issues. Disease-related factors did not predict executive performance, with family functioning and sleep problems emerging as important factors for executive development. The findings from this thesis inform future research in early assessment and intervention.
Supervisor: Kirkham, F. J. K. ; de Haan, M. Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available